Clinicopathologic profile of hypopigmented mycosis fungoides in India.

BACKGROUND: Hypopigmented mycosis fungoides (HMF) is an under recognized disease in India, which is often mistaken for Hansen disease or vitiligo, resulting in delayed diagnosis and treatment. AIM: To describe the clinical, histopathologic and immunohistochemical features of HMF in Indian patients. MATERIALS AND METHODS: All cases presenting as hypopigmented lesions that were signed out as MF between 2001 and 2009 (15 cases) were included. Clinical data and histopathology slides were reviewed. Immunostains for CD4, CD8, and CD1a were done, where tissue was available. RESULTS: The age ranged from 14 to 38 years with a male preponderance. The commonest presentation was multiple hypopigmented patches on limbs and trunk with the duration of the lesions varying from 4 months to 14 years. All cases showed a psoriasiform/lichenoid epidermal pattern, disproportionate epidermotropism, basilar tagging of lymphocytes, monomorphous lymphocytes, haloed lymphocytes, and wiry dermal collagen. Other important findings were infiltration of hair follicles, larger epidermal lymphocytes, atypia of dermal lymphocytes, and stuffed dermal papillae. Dermal edema was absent in all cases. Immunohistochemistry done on 10 cases showed a CD8 phenotype in 6 cases and CD4 phenotype in the remaining 4 cases. CONCLUSIONS: Histopathology supplemented by immunohistochemistry is reliable in making a diagnosis of HMF. It is important to be aware of this uncommon, yet significant disease.

[Creole dyschromia or idiopathic macular hypomelanosis of the melanodermic halfcast of Guillet-Hélénon]. / Dyschromie créole ou hypomélanose maculeuse idiopathique du métis mélanoderme de Guillet-Hélénon.

Melanodermic halfcasts may develop an original cutaneous dyschromia known as "progressive and extensive hypomelanosis" (Guillet-Helenon 1988). This disease is characterized by hypochromic and coalescent macules on the back and abdomen with possible spontaneous improvement within five years, favoured by UV exposure. The disease is not restricted to a limited geographic group: eight observations were collected in melanodermic patients leaving in temperate area. The pathogenesis of the disorder involves a variation in melanosome size and distribution with decrease in production of type IV melanosomes featuring a change of ultrastructural phenotype of melanogenesis. Since it may be misdiagnosed as fungal disease, leprosy or achromic eczema leading to useless laboratory examinations, this specific and frequent disease deserves to be known and recognized.